Neurophysiological findings in the hereditary presenile dementia characterised by polycystic lipomembranous osteodysplasia and sclerosing leukoencephalopathy.
نویسندگان
چکیده
17 patients suffering from the presenile dementia characterised by polycystic lipomenbranous osteodysplasia and sclerosing leukoencephalopathy were studied neurophysiologically. Noteworthy findings were diffuse slowing, the accentuation of 6-8 Hz parasagittal activity, slowing of the dominant occipital rhythm and, in the late phase when 13 of patients got epileptic seizures, there was paroxysmal activity in the EEG.
منابع مشابه
Word List Learning in Patients with Polycystic Lipomembranous Osteodysplasia with Sclerosing Leukoencephalopathy
BACKGROUND/AIMS Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) is a rare hereditary disease that is characterized by a combination of progressive presenile dementia and sclerosing leukoencephalopathy with bone cysts. No quantitative information on verbal memory functioning in PLOSL patients compared with control subjects is available. METHODS 23 patients ...
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The combination of bilateral lytic lesions in the bones of the lower and upper extremities and presenile dementia is characteristic of polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy, also known as Nasu-Hakola disease. The clinical course of this rare and fatal disorder is characterized by pathologic fractures of these often painful lesions, rapid progression of dem...
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Nasu-Hakola disease (NHD, polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy, PLOSL) is a recessively inherited disorder characterized by systemic bone cysts and progressive presenile dementia associated with sclerosing encephalopathy. The disease has a worldwide distribution, but most patients have been reported in Finland and in Japan; in Italy there are anecdotal re...
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Nasu-Hakola disease (NHD-polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy; PLOSL) is a rare autosomal recessive disorder, caused by mutations in two genes: TREM 2 and DAP 12. NHD is characterized by a combination of diffuse bone cysts and pre-senile dementia. Most of the NHD cases first present in early adulthood with skeletal abnormalities (osseous phase). Neurologi...
متن کامل[Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy].
BACKGROUND Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (Nasu-Hakola syndrome) (PLO-SL) is an unusual cause of early dementia in which the decline in cognitive function is associated with multiple bone cysts, in particular in hands and feet. About 165 cases of this autosomal recessive hereditary disease have been reported worldwide, mostly from Finland and Japan....
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عنوان ژورنال:
- Journal of neurology, neurosurgery, and psychiatry
دوره 46 6 شماره
صفحات -
تاریخ انتشار 1983